Hirschsprung's disease

From 1972 to 1985, 214 patients were treated for Hirschsprung's disease. One patient underwent a Swenson pull-through operation, 13 a Soave procedure, and 195 had modifications of the Duhamel procedure. Six children are newly diagnosed and awaiting definitive therapy. Of the patients in whom a modified Duhamel procedure was performed, 153 were males (78%) and 7 (4%) had Down's syndrome. There was no operative mortality for the procedure, and only 4 late deaths have been observed (3 with Down's syndrome). Fecaloma occurred in 12 of 14 patients who underwent the classic operation described by Duhamel, and postoperative enterocolitis was noted in 4 of 6 patients who had Martin's long Duhamel modification for total colonic aganglionosis. No urologic problems or anastomotic leaks were identified, and only 1 patient developed a rectal stricture postoperatively. Growth and development and fecal continence have been excellent in the vast majority of patients. The modified Duhamel pull-through operation is associated with excellent survival and highly acceptable long-term function.Offprint requests to: D. W. Vane at the above address